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Outcomes Of Surgical Intervention On Primary Pulmonary Vein Obstruction
Joshua M Rosenblum, Firat Altin, Kirk Kanter, Scott Gillespie, *Raina Sinha, Melinda Cory, Brian Kogon, *Bahaaldin Alsoufi
Emory University School of Medicine, Atlanta, GA

Objectives:
Primary pulmonary vein obstruction (PPVO) is increasingly diagnosed in children with no prior pulmonary vein intervention history. Surgical management of PPVO is challenging and associated with high recurrence and mortality rates. We aim to describe characteristics of patients who underwent surgery for PPVO at our center, and examine factors associated with treatment failures.
Methods:
A retrospective review of all patients who underwent surgery for PPVO was completed (2002-16). Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial septum were excluded. Regression analyses were performed to examine patients / anatomic characteristics and surgical details associated with treatment failures (mortality, recurrent obstruction and re-intervention).
Results:
Thirty-four children including 14 males (41%) underwent surgery for PPVO at a median age of 8.9 months (IQR 0.48 1.68 years). Median number of affected veins was 2 (range 1-4) and they were on the left (n=25, 73%), right (n=6, 18%) or both sides (n=3, 9%). Four patients (12%) had pulmonary vein atresia while the remaining 30 (88%) had stenosis with median pre-operative gradient of 10 mm Hg (IQR 6-12). The majority of patients (n=28, 82%) had sutureless pericardial well repair technique.
There were 3 hospital deaths (9%) and 3 late deaths (9%). Fourteen patients (41%) developed recurrent obstruction at a median of 6.25 months following surgery and 7 (20%) underwent pulmonary vein re-intervention. On competing risks analysis, 1 year from pulmonary vein surgery, 9% had died, 27 % had undergone re-intervention, and 64% were alive without re-intervention. At 5 years, the numbers were 9%, 45% and 46%, respectively. [Figure] Overall survival at 1 and 5 years was 88% and 80%.
On regression analyses, factors associated with pulmonary vein re-intervention were bilateral disease (HR 5.65 (1.07-29.77) p=0.041) and the number of veins involved (HR 3.25 (1.25-8.40) p=0.015). Similarly, factors associated with overall mortality were bilateral disease (HR 5.65 (1.03-30.84) p=0.046) and the number of veins involved (HR 5.65 (1.03-30.84) p=0.046).
Conclusions:
Surgical management of PPVO is challenging and associated with significant obstruction recurrence risk. Patients with multiple and / or bilateral pulmonary vein disease are at higher risk of recurrent obstruction and subsequently death, with the majority of those failures occurring within the first year following initial repair. Given these disappointing results, multi-disciplinary management strategy of those patients that combines attentive surgical, interventional and imaging efforts, and the experimental use of adjunctive medications need to be attempted to alter the unfavorable course of this disease. Given limited single-institutions experience in this pathology, collaborative multi-institutional efforts might be warranted.


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